Symptomatic obstructive hydrocephalus caused by choroid plexus hyperplasia in a pediatric patient: illustrative case

Ana Sofia Alvarez Department of Neurosurgery, Fundacion Jimenez Diaz University Hospital, Madrid, Spain

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John P McGinnis Department of Neurosurgery, Baylor College of Medicine, Houston, Texas; and

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Rajan Patel Department of Neurosurgery, Baylor College of Medicine, Houston, Texas; and

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Howard L Weiner Department of Neurosurgery, Division of Pediatric Neurosurgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas

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BACKGROUND

Choroid plexus hyperplasia has been described as a rare cause of communicating hydrocephalus due to cerebrospinal fluid (CSF) overproduction. However, this is the first report of symptomatic obstructive hydrocephalus caused by mechanical obstruction of the aqueduct by a hyperplastic choroid plexus.

OBSERVATIONS

A 4-year-old male presented with headaches and intermittent emesis. Magnetic resonance imaging (MRI) of the brain showed abnormal enlargement of the choroid plexus in the lateral ventricles with extension into the third ventricle, resulting in obstruction of the aqueduct of Sylvius, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy (ETV) was chosen as the surgical treatment. During the procedure, high pressure flow of clear CSF was noted. Normal intraventricular anatomy was identified, and no cyst or tumor was found. The postoperative MRI showed a patent third ventriculostomy, without complication, and a significant decrease in supratentorial ventricular size. The patient was discharged 3 days after surgery with a complete resolution of symptoms.

LESSONS

Choroid plexus hyperplasia has the potential to cause obstructive hydrocephalus, and it can be effectively treated with ETV. Our hypothesis is that the change in pressure caused by the procedure may have led to an uncorking of the aqueduct by the hyperplastic choroid plexus, contributing to the observed improvement.

ABBREVIATIONS

CSF = cerebrospinal fluid; DVHCP = diffuse villous hyperplasia of the choroid plexus; ETV = endoscopic third ventriculostomy; MRI = magnetic resonance imaging

BACKGROUND

Choroid plexus hyperplasia has been described as a rare cause of communicating hydrocephalus due to cerebrospinal fluid (CSF) overproduction. However, this is the first report of symptomatic obstructive hydrocephalus caused by mechanical obstruction of the aqueduct by a hyperplastic choroid plexus.

OBSERVATIONS

A 4-year-old male presented with headaches and intermittent emesis. Magnetic resonance imaging (MRI) of the brain showed abnormal enlargement of the choroid plexus in the lateral ventricles with extension into the third ventricle, resulting in obstruction of the aqueduct of Sylvius, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy (ETV) was chosen as the surgical treatment. During the procedure, high pressure flow of clear CSF was noted. Normal intraventricular anatomy was identified, and no cyst or tumor was found. The postoperative MRI showed a patent third ventriculostomy, without complication, and a significant decrease in supratentorial ventricular size. The patient was discharged 3 days after surgery with a complete resolution of symptoms.

LESSONS

Choroid plexus hyperplasia has the potential to cause obstructive hydrocephalus, and it can be effectively treated with ETV. Our hypothesis is that the change in pressure caused by the procedure may have led to an uncorking of the aqueduct by the hyperplastic choroid plexus, contributing to the observed improvement.

ABBREVIATIONS

CSF = cerebrospinal fluid; DVHCP = diffuse villous hyperplasia of the choroid plexus; ETV = endoscopic third ventriculostomy; MRI = magnetic resonance imaging

Choroid plexus hyperplasia has been described as a rare cause of communicating hydrocephalus due to cerebrospinal fluid (CSF) overproduction. However, this is the first report of a case of symptomatic obstructive hydrocephalus caused by choroid plexus hyperplasia.

Illustrative Case

A 4-year-old male with 1-year history of headaches and intermittent emesis was brought to the emergency department because of a progressive worsening of symptoms. Computed tomography showed moderate dilation of the supratentorial ventricular system. The patient was admitted to the pediatric neurosurgery service for observation and further workup. Magnetic resonance imaging (MRI) of the brain showed abnormal asymmetrical enlargement of the choroid plexus in the right atrium with extension into the third ventricle and obstruction of the aqueduct of Sylvius (Figs. 1 and 2). In addition, associated obstructive hydrocephalus of the lateral and third ventricles without transependymal periventricular CSF flow was observed. Differential radiological considerations included choroid plexus papilloma, choroid plexus carcinoma, or villous choroid plexus hyperplasia. The radiologist was concerned about a possible tumor. MRI of the spine did not show any significant findings.

FIG. 1
FIG. 1

Preoperative postcontrast sagittal T1-weighted MRI showing obstruction of the aqueduct of Sylvius by the choroid plexus.

FIG. 2
FIG. 2

Preoperative postcontrast axial T1-weighted MRI showing supratentorial hydrocephalus.

Given the clinical and radiological findings, endoscopic third ventriculostomy (ETV) was indicated. The surgery was performed through a standard right frontal burr hole, and intraoperative ultrasound guidance was used to plan the trajectory into the anterior horn of the right lateral ventricle. High pressure flow of clear CSF was noted after initial placement of the ventricular peel-away sheath needed for insertion of the endoscope. CSF samples were collected and sent for analysis. The procedure was then performed with a flexible endoscope. We were able to visualize the normal intraventricular anatomy, including the right foramen of Monro. In the third ventricle, we visualized the mammillary bodies and the infundibular recess. The floor of the third ventricle was noted to be extremely thinned. With blunt dissection, we fenestrated the floor of the third ventricle just posterior to the infundibular recess and anterior to the basilar artery, which was easily seen. We then drove the endoscope through the fenestration and were able to visualize the interpeduncular cistern in the subarachnoid space with a clear view of the brainstem and basilar artery and its perforating branches. There was excellent CSF flow through the fenestration. We then drove the endoscope to visualize the posterior third ventricle. No cyst or tumor was obstructing the aqueduct, and the anatomy was completely normal. After the ETV was performed, we observed a repositioning of the choroid plexus, which was previously obstructing the aqueduct. Subsequently, it was found to be located more superiorly within the third ventricle, no longer obstructing the aqueduct. However, it is worth noting that it may still have the potential for intermittent obstruction. Although there was a possibility of intermittent obstruction of the aqueduct in the future, considering that the ETV was performed successfully, we decided not to coagulate the hyperplastic choroid plexus because of the associated risk of bleeding. The endoscope was then removed as the peel-away sheath was gently withdrawn, and the procedure ended without complications.

The patient tolerated the procedure well with an uneventful postoperative course. The results of the CSF analysis, including cytology and microbiological studies, were normal. Postoperative MRI (Fig. 3) showed the third ventriculostomy with an associated significant decrease in supratentorial ventricular size. The patient was discharged 3 days after the procedure with a complete resolution of symptoms.

FIG. 3
FIG. 3

Postoperative postcontrast sagittal T1-weighted MRI showing resolution of the aqueduct of Sylvius obstruction and a patent third ventriculostomy.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

We present a pediatric patient with asymmetrical choroid plexus hyperplasia who developed symptomatic hydrocephalus caused by mechanical obstruction of the aqueduct by the hyperplastic choroid plexus.

Choroid plexus hyperplasia is a rare condition involving enlargement of a histologically normal choroid plexus. Diffuse villous hyperplasia of the choroid plexus (DVHCP) was first reported in 1924 by Loyal E. Davis,1 who described hypertrophied choroid plexus with an increased number of epithelial cuboidal cells, which remained normal in shape and size. Even though this entity may have sometimes been diagnosed with choroid plexus papillomas,2 the term DVHCP is used for referring to the diffuse enlargement of an otherwise histologically normal choroid plexus. Unlike in choroid plexus hyperplasia, choroid plexus papilloma has a neoplastic nature and forms a mass lesion in which its cells have a higher proliferation index.3 MRI of choroid plexus hyperplasia demonstrates a diffuse enlargement and homogeneous enhancement of the choroid plexus without any discrete masses.

DVHCP is an uncommon benign disorder, primarily observed in pediatric patients, and can cause an overproduction of CSF leading to communicating hydrocephalus. Several case reports in the pediatric neurosurgical literature have described children presenting with DCPH, wherein the choroid plexus tissue appears morphologically normal but congenitally enlarged, leading to an excessive production of CSF and communicating hydrocephalus in early life.5,6 The primary treatment for this condition typically involves the use of a ventriculoperitoneal shunt.

In a very few cases, this condition has been reported in adults.4 The hypothesis explaining its occurrence in adulthood suggests that it may represent a milder form of DCPH, wherein enlargement of the choroid plexus occurs congenitally but becomes symptomatic later in life due to decreased brain compliance and an inability to compensate for excessive CSF flow.

To the best of our knowledge, this is the first time a case of symptomatic obstructive hydrocephalus caused by choroid plexus hyperplasia has been reported.

Lessons

In this case report, we present a patient with choroid plexus hyperplasia who experienced obstructive hydrocephalus. We successfully performed an ETV, which resulted in significant improvement in the hydrocephalus, along with displacement of the choroid plexus from the aqueduct. Our hypothesis is that the CSF flow through the ventriculostomy caused a pressure shift that may have led to an uncorking of the aqueduct by the hyperplastic choroid plexus, contributing to the observed improvement of symptoms.

Author Contributions

Conception and design: Alvarez, McGinnis, Weiner. Acquisition of data: Alvarez, McGinnis, Weiner. Analysis and interpretation of data: Alvarez, McGinnis. Drafting the article: Alvarez, Weiner. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Alvarez. Administrative/technical/material support: McGinnis.

References

  • 1

    Davis LE. A physio-pathologic study of the choroid plexus with the report of a case of villous hypertrophy. J Med Res. 1924;44(5):521534.11.

  • 2

    Ray BS, Peck FC Jr. Papilloma of the choroid plexus of the lateral ventricles causing hydrocephalus in an infant. J Neurosurg. 1956;13(4):317322.

  • 3

    D’Ambrosio AL, O’Toole JE, Connolly ES Jr, Feldstein NA. Villous hypertrophy versus choroid plexus papilloma: a case report demonstrating a diagnostic role for the proliferation index. Pediatr Neurosurg. 2003;39(2):9196.

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  • 4

    Cox JT, Gaglani SM, Jusué-Torres I, et al. Choroid plexus hyperplasia: a possible cause of hydrocephalus in adults. Neurology. 2016;87(19):20582060.

  • 5

    Aziz AA, Coleman L, Morokoff A, Maixner W. Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children? Pediatr Radiol. 2005;35(8):815818.

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    • Search Google Scholar
    • Export Citation
  • 6

    Fujimoto Y, Matsushita H, Plese JP, Marino R Jr. Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature. Pediatr Neurosurg. 2004;40(1):3236.

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    • Search Google Scholar
    • Export Citation
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  • FIG. 1

    Preoperative postcontrast sagittal T1-weighted MRI showing obstruction of the aqueduct of Sylvius by the choroid plexus.

  • FIG. 2

    Preoperative postcontrast axial T1-weighted MRI showing supratentorial hydrocephalus.

  • FIG. 3

    Postoperative postcontrast sagittal T1-weighted MRI showing resolution of the aqueduct of Sylvius obstruction and a patent third ventriculostomy.

  • 1

    Davis LE. A physio-pathologic study of the choroid plexus with the report of a case of villous hypertrophy. J Med Res. 1924;44(5):521534.11.

  • 2

    Ray BS, Peck FC Jr. Papilloma of the choroid plexus of the lateral ventricles causing hydrocephalus in an infant. J Neurosurg. 1956;13(4):317322.

  • 3

    D’Ambrosio AL, O’Toole JE, Connolly ES Jr, Feldstein NA. Villous hypertrophy versus choroid plexus papilloma: a case report demonstrating a diagnostic role for the proliferation index. Pediatr Neurosurg. 2003;39(2):9196.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Cox JT, Gaglani SM, Jusué-Torres I, et al. Choroid plexus hyperplasia: a possible cause of hydrocephalus in adults. Neurology. 2016;87(19):20582060.

  • 5

    Aziz AA, Coleman L, Morokoff A, Maixner W. Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children? Pediatr Radiol. 2005;35(8):815818.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Fujimoto Y, Matsushita H, Plese JP, Marino R Jr. Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature. Pediatr Neurosurg. 2004;40(1):3236.

    • PubMed
    • Search Google Scholar
    • Export Citation

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